Figures
- Fig. 1—
High-resolution computed tomography from case No. 1 shows diffuse, numerous, small rounded lung cysts involving the upper lobes and superior segment of the left lower lobe. In distinction to centrilobular emphysema, the cysts have well-defined walls.
- Fig. 2—
High-resolution computed tomography from case No. 2 shows bilateral asymmetric bullous disease.
- Fig. 3—
High-resolution computed tomography from case No. 3 shows scattered small, round lung cysts in both upper lobes.
Tables
- Table 1—
New cases of neurofibromatosis with diffuse lung disease
Case No. Clinical Pulmonary function HRCT Age yrs Sex Smoker FVC FEV1 Ratio DL,CO 1 66 Male Former 4.7 (98) 2.9 (78) 0.61 8.24 (24) Upper lobe predominance of thin-walled cysts (fig. 1) 2 53 Male Former 4.2 (95) 1.3 (41) 0.43 5.7 (15) Bilateral asymmetric bullae (fig. 2) 3 70 Female Never N/A N/A N/A N/A Scattered upper lobe cysts (fig. 3) Data are presented as n or n (%). HRCT: high-resolution computed tomography; FVC: forced vital capacity; FEV1: forced expiratory volume in one second; DL,CO: diffusing capacity of the lung for carbon monoxide; N/A: not applicable.
- Table 2—
Demographic, clinical and laboratory data
Variable Subjects n 64 Demographics Age yrs* 50 (23–72) Male/female 44/20 Symptoms# Dyspnoea 28 (80) Cough 11 (32) Chest pain 2 (5) Asymptomatic 4 (11) Pulmonary function pattern¶ Normal 1 (3) Obstructive 13 (43) Restrictive 11 (37) Mixed 5 (17) Low DL,CO+ 17 (94) Data are presented as n, mean (range) or n (%). DL,CO: diffusing capacity of the lung for carbon monoxide. #: n = 35; ¶: n = 30; +: n = 18.
- Table 3—
Radiographic data
Modality Chest radiograph# Mottled 20 (32) Linear densities 38 (61) Nodular densities 13 (13) Bullae 46 (73) Bullae upper localisation 41 (93) Honeycombing 7 (12) HRCT¶ Ground glass 3 (37) Reticular abnormality 4 (50) Bullae 4 (50) Emphysema 2 (25) Cyst 2 (25) Honeycombing 0 (0) Data are presented as n (%). HRCT: high-resolution computed tomography. #: n = 63; ¶: n = 8.
- Table 4—
Comparison of smokers and nonsmokers
Variable Smokers Nonsmokers p-value Subjects n 12 4 Demographic Age yrs 52.4±9.2 46.75±20.5 0.4 Male/female 9/3 (75/25) 0/4 (0/100) 0.01 Pulmonary function testing 7 2 Obstructive pattern 7 (100) 2 (100) 0.5 Chest radiograph 12 3 Linear densities 10 (90) 2 (50) 0.4 Bullae 11 (91) 0 (0) 1.0 HRCT 6 2 Ground-glass opacities 3 (50) 0 (0) 0.5 Reticular 3 (50) 1(50) 1.0 Bullae 3(50) 1(50) 1.0 Cyst 1(20) 1(50) 1.0 Emphysema 2(50) 0(00) 1.0 Data are presented as n, mean±SD or n (%). HRCT: high-resolution computed tomography. Surgical lung biopsy results were available in 14 cases, all 14 showed fibrosis and 13 showed interstitial inflammation (93%). Honeycomb cysts were present in three cases (23%).
